title: Detection of anticonductive tissue autoantibodies in a patient with chronic intestinal pseudo-obstruction and sick sinus syndrome.
abstract: A 26-year-old patient was diagnosed as having chronic intestinal pseudo-obstruction with manometric and histopathologic features suggestive of an intestinal myopathy. Histology was characterized by smooth muscle degeneration without inflammatory or immune cells. The severe gut dysfunction required full parenteral nutritional support. After a few months, the patient developed symptomatic tachy-brady arrhythmia episodes with syncopes. A thorough diagnostic work-up led to a diagnosis of sick sinus syndrome, which was managed by pacemaker implantation and administration of β-blockers. This led to a partial improvement in tachy-brady arrhythmia episodes. Nonetheless, the patient continued to experience sustained supraventricular tachyarrhythmia runs, poorly responsive to increasing β-blocker doses. To investigate the origin of the cardiologic impairment, the patient was tested for anticonductive tissue autoantibodies, which were positive, thus supporting a possible autoimmune origin of the dysrhythmia. Other autoantibodies were negative. On the basis of these findings, the patient was treated with high-dose steroids, which were then tapered. The patient responded to the steroid treatment and did not experience further episodes of syncope and tachyarrhythmias. The severe gut dysfunction remained unchanged. This case highlights an association between severe gut dysfunction and cardiac conductive tissue abnormalities, with autoantibodies to conductive tissue possibly causing the dysrhythmia. The severe gut and heart (likely autoimmune-mediated) dysfunction presented in this case provides a basis to further assess a link between intestinal and cardiac abnormal rhythmicity.
authors.affiliation: aDepartment of Medical and Surgical Sciences/Digestive Diseases and Internal Medicine, St. Orsola-Malpighi Hospital bDepartment of Medical and Veterinary Sciences, University of Bologna, Bologna cDepartment of Internal Medicine, Division of Cardiology, San Giovanni Battista Hospital, University of Turin, Turin, Italy dEnteric NeuroScience Program, Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, Minnesota, USA.
authors.forename: Giacomo
authors.initials: G
authors.lastname: Caio
authors.forename: Umberto
authors.initials: U
authors.lastname: Volta
authors.forename: Enrico
authors.initials: E
authors.lastname: Cerrato
authors.forename: Paolo
authors.initials: P
authors.lastname: Clavenzani
authors.forename: Nicolò
authors.initials: N
authors.lastname: Montali
authors.forename: Rosanna
authors.initials: R
authors.lastname: Cogliandro
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Stanghellini
authors.forename: Pier Giorgio
authors.initials: PG
authors.lastname: Golzio
authors.forename: Fiorenzo
authors.initials: F
authors.lastname: Gaita
authors.forename: Gianrico
authors.initials: G
authors.lastname: Farrugia
authors.forename: Roberto
authors.initials: R
authors.lastname: De Giorgio
mesh_terms: D000328:Adult; D001323:Autoantibodies / Q000097:blood*; D001327:Autoimmune Diseases / Q000276:immunology*; D005938:Glucocorticoids / Q000627:therapeutic use; D006329:Heart Conduction System / Q000276:immunology*; D006801:Humans; D007418:Intestinal Pseudo-Obstruction / Q000150:complications / Q000276:immunology* / Q000473:pathology; D008297:Male; D009130:Muscle, Smooth / Q000473:pathology; D012804:Sick Sinus Syndrome / Q000150:complications / Q000188:drug therapy / Q000276:immunology*
chemical_list: D001323:Autoantibodies; D005938:Glucocorticoids